07 June 2022
: Case report
Thrombotic Microangiopathy Secondary to Systemic Sclerosis with Severe Complement Activation Not Responsive to Eculizumab: A Case Report
Unusual clinical course, Challenging differential diagnosis, Unusual or unexpected effect of treatment, Diagnostic / therapeutic accidents, Unexpected drug reaction
Masahiko Nakamura1ABCDEF*, Tomohiro Abe
DOI: 10.12659/AJCR.936116
Am J Case Rep 2022; 23:e936116
Figure 2. Clinical course and the values of thrombotic microangiopathy. On admission, the patient showed a low platelet count (red line), increased levels of creatinine (yellow line), mildly elevated lactate dehydrogenase (blue line), and reduced urine volume (blue column). We introduced continuous hemodiafiltration (CHDF) on day 1. Plasma exchange (brown bars) was initiated at day 3, for 24 times. Because the platelet count, creatinine levels, and urine volume did not change in response to plasma exchange, we administered 900 mg of eculizumab on days 7 and 14 (red arrow). After the initiation of eculizumab, the platelet count, creatinine levels, and urine volume did not recover. We administered 1 g of mPSL for 3 days on day 18, followed by 20 mg of PSL per day. We transfused 30 units of platelet concentrate on day 42. On day 43, the patient was diagnosed with thrombotic microangiopathy secondary to systemic sclerosis. We performed repeated plasma exchanges on day 64. CHDF was discontinued on day 55, and intermittent HD was performed from day 56. Subsequently, platelet count and creatinine levels were controlled. Because urine volume was also controlled, intermittent hemodialysis was terminated on day 151. CHDF – continuous hemodiafiltration; HD – hemodialysis; PEX – plasma exchange; mPSL – methylprednisolone; PSL – prednisolone; LDH – lactate dehydrogenase; PLT – platelet count; CRE – creatinine.